AKT1

Chr 14AD

AKT serine/threonine kinase 1

Also known as: AKT, PKB, PKB-ALPHA, PRKBA, RAC, RAC-ALPHA

AKT1 encodes a serine/threonine protein kinase that regulates cellular metabolism, proliferation, survival, and growth through phosphorylation of downstream substrates in the PI3K/AKT signaling pathway. Mutations cause Proteus syndrome and Cowden syndrome 6, both presenting with excessive tissue growth and increased cancer risk, with inheritance following an autosomal dominant pattern. AKT1 is highly constrained against loss-of-function variants (pLI 0.98), consistent with its essential role in cellular homeostasis.

OMIMResearchSummary from RefSeq, OMIM, UniProt
GOFmechanismADLOEUF 0.325 OMIM phenotypes
Clinical SummaryAKT1
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Gene-Disease Validity (ClinGen)
Cowden syndrome 6 · ADLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.98). One damaged copy is likely sufficient to cause disease.
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Clinical Trials
12 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint
0.32LOEUF
pLI 0.976
Z-score 4.31
OE 0.14 (0.070.32)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
3.48Z-score
OE missense 0.46 (0.400.53)
152 obs / 329.7 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios
LoF OE0.14 (0.070.32)
00.351.4
Missense OE0.46 (0.400.53)
00.61.4
Synonymous OE1.05
01.21.6
LoF obs/exp: 4 / 29.1Missense obs/exp: 152 / 329.7Syn Z: -0.42
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveAKT1-related Proteus syndromeGOFAD
DN
0.6646th %ile
GOF
0.83top 10%
LOF
0.66top 25%

This gene has evidence for multiple mechanisms of pathogenicity (loss-of-function, gain-of-function and dominant-negative). The Badonyi & Marsh model scores gain-of-function highest among its predictions, but genomic evidence (constraint, ClinVar variant spectrum, and literature) most strongly supports loss-of-function (haploinsufficiency). Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

LOFprediction above median · LOEUF 0.32
GOFprediction above median · 1 literature citation
DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Literature Evidence

GOFThe gain-of-function mutation in the pleckstrin homology domain of AKT1 (AKT1E17K) occurs in lung and breast cancer.PMID:35681625

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

AKT1 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

Lymphoma, Non-HodgkinMultiple MyelomaAdvanced Solid Tumors

Canadian Profiling and Targeted Agent Utilization Trial (CAPTUR)

RECRUITING
NCT03297606Phase PHASE2Canadian Cancer Trials GroupStarted 2018-03-23
OlaparibDasatinibNivolumab plus Ipilimumab
Metastatic Thyroid Gland CarcinomaUnresectable Thyroid Gland Carcinoma

Dabrafenib and Lapatinib in Treating Patients With Refractory Thyroid Cancer That Cannot Be Removed by Surgery

ACTIVE NOT RECRUITING
NCT01947023Phase PHASE1National Cancer Institute (NCI)Started 2013-09-27
Biopsy ProcedureBiospecimen CollectionComputed Tomography
Fallopian Tube Endometrioid AdenocarcinomaFallopian Tube High Grade Serous AdenocarcinomaOvarian Endometrioid Adenocarcinoma

Testing the Addition of Ipatasertib to the Usual Chemotherapy Treatment (Paclitaxel and Carboplatin) for Stage III or IV Epithelial Ovarian Cancer

ACTIVE NOT RECRUITING
NCT05276973Phase PHASE1National Cancer Institute (NCI)Started 2022-09-08
BiopsyCarboplatinIpatasertib
Breast CancerER-positive Breast CancerHER2-negative Breast Cancer

Levels of Circulating Tumor DNA as a Predictive Marker for Early Switch in Treatment for Patients With Metastatic (Stage IV) Breast Cancer

ACTIVE NOT RECRUITING
NCT05826964Phase PHASE2University of MiamiStarted 2023-06-12
AI+CDK4/6iSERD+CDK4/6imTOR inhibitor + AI
Malignant Solid Neoplasms

Adapting Treatment to the Tumor Molecular Alterations for Patients With Advanced Solid Tumors: MyOwnSpecificTreatment

RECRUITING
NCT02029001Phase PHASE2Centre Leon BerardStarted 2014-03
Nilotinib (400 mg BID)Everolimus (10 mg QD)Sorafenib (400 mg BID)
Hormone Receptor(HR)-Positive, Low HER2 Advanced Breast Cancer Patients (HER2 IHC 1+ or 2+ & ISH Negative)

Trastuzumab Deruxtecan vs Endocrine Therapy in Low-HER2 HR+ Advanced Breast Cancer

RECRUITING
NCT06837792Phase PHASE2Yonsei UniversityStarted 2023-10-01
Experimental treatment armControl treatment arm
HR+/HER2-negative Breast CancerMetastatic Breast Cancer

Evexomostat Plus PI3K or AKT Inhibitor and Fulvestrant in Patients With a PI3K Alteration and HR+/Her2- Breast Cancer

RECRUITING
NCT05455619Phase PHASE1, PHASE2SynDevRx, Inc.Started 2022-08-26
Evexomostat
Triple Negative Breast CancerBreast Cancer

Serial Circulating Tumor DNA (ctDNA) Monitoring During Adjuvant Capecitabine in Early Triple-negative Breast Cancer

RECRUITING
NCT04768426Phase PHASE2Stanford UniversityStarted 2021-02-03
Capecitabine
Locally Advanced (Inoperable) or Metastatic Breast Cancer

Capivasertib + CDK4/6i + Fulvestrant for Advanced/Metastatic HR+/HER2- Breast Cancer (CAPItello-292)

RECRUITING
NCT04862663Phase PHASE3AstraZenecaStarted 2021-05-10
CapivasertibFulvestrantPalbociclib
Advanced Solid TumorsBreast CancerBreast Carcinoma

A Phase 1 Study of ATV-1601 in Patients With Advanced Cancer That Have AKT1 E17K Mutations

ACTIVE NOT RECRUITING
NCT07038369Phase PHASE1Atavistik Bio, IncStarted 2025-07-29
ATV-1601ATV-1601 + Fulvestrant
Lymphatic MalformationLymphatic AbnormalityLymphangioma

Institution of an Italian Registry and Biobank for Biological Sample Collection

RECRUITING
NCT06892964Phase NAFondazione Policlinico Universitario Agostino Gemelli IRCCSStarted 2024-06-27
Genetic profiling of all eligible patients
Patients With HER2-positive Breast Cancer (BC) Suitable for Neoadjuvant Therapy

A Multicenter Phase III Clinical Study and Translational Research on Adaptive Neoadjuvant Therapy of De-escalation and Escalation for HER2-Positive Breast Cancer Based on Multi-Omics Response Evaluation Model

NOT YET RECRUITING
NCT07239271Phase PHASE3Cancer Institute and Hospital, Chinese Academy of Medical SciencesStarted 2025-11-16
The control groupAdaptive therapy of de-escalation and escalation based on imaging and molecular analysis for predicting response
Clinical Literature
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